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acquired von willebrand disease treatment

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https://www.readbyqxmd.com/read/30401518/management-of-acquired-von-willebrand-syndrome
#1
Janie Charlebois, Georges-Étienne Rivard, Jean St-Louis
Acquired von Willebrand syndrome (AVWS) is a rare acquired bleeding disorder that resembles von Willebrand disease by its clinical symptoms and laboratory findings, but differs by its negative personal and family history of bleeding diathesis. AVWS is mostly seen in the elderly, but it has been described in children, often in those with congenital heart disease and Wilms tumor. It is most commonly associated with lymphoproliferative, myeloproliferative, cardio-vascular, or autoimmune diseases, solid tumors, and certain drugs...
October 30, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/30225530/administration-of-plasma-derived-coagulation-factor-viii-during-the-perioperative-period-of-mastectomy-for-breast-cancer-with-acquired-von-willebrand-syndrome
#2
Ritsuko Sasaki, Yoshiya Horimoto, Ju Mizuno, Yoko Edahiro, Tsukasa Ohmori, Norio Komatsu, Mitsue Saito
BACKGROUND: Acquired von Willebrand syndrome (aVWS) is a rare bleeding disorder with laboratory findings similar to those of congenital von Willebrand disease (VWD). Patients with aVWS may require prophylactic treatment to prevent excessive bleeding following surgery. To our knowledge, to date, there have been no reports on perioperative management for breast cancer patients with aVWS. CASE PRESENTATION: A 60-year-old woman with breast cancer was diagnosed with aVWS due to polycythemia vera...
September 17, 2018: Surgical Case Reports
https://www.readbyqxmd.com/read/30214173/von-willebrand-disease-in-the-elderly-clinical-perspectives
#3
REVIEW
John Chapin
Von Willebrand disease (VWD) is an inherited bleeding disorder that affects up to 1% of the population. In most cases, VWD results from a mutation in the von Willebrand Factor (VWF) gene, which alters the amount and function of VWF, a key glycoprotein in both primary and secondary hemostasis. A comprehensive analysis of patients with VWD should include VWF activity, antigen levels, platelet function, and a careful bleeding history. Treatment options include antifibrinolytics, desmopressin, and VWF replacement therapy...
2018: Clinical Interventions in Aging
https://www.readbyqxmd.com/read/30048974/understanding-left-ventricular-assist-devices
#4
REVIEW
Nadia Aissaoui, Jerome Jouan, Melissa Gourjault, Benoit Diebold, Sofia Ortuno, Amer Hamdan, Christian Latremouille, Romain Pirracchio, Michiel Morshuis
BACKGROUND/AIMS: Long-term mechanical assist devices are now commonly used in the treatment of severe heart failure to unload the failing ventricle, maintain sufficient end-organ perfusion and improve functional capacity. Depending on the assisted ventricles, 3 categories of long-term assist devices are available: left ventricular assist device (LVAD), biventricular assist device and total artificial heart. Improvements in technology, especially the advent of smaller, durable continuous flow pumps, have led to the use of LVADs in a much broader population of patients in the last 10 years...
2018: Blood Purification
https://www.readbyqxmd.com/read/29984746/combined-coagulopathy-can-induce-both-hemorrhagic-and-thrombotic-complications-in-multiple-myeloma
#5
Ichiro Kawashima, Katsuhiro Takano, Takuma Kumagai, Megumi Koshiishi, Saori Oishi, Yuki Sueki, Kei Nakajima, Toru Mitsumori, Keita Kirito
Coagulation abnormalities are a rare but critical complication associated with plasma cell diseases. We herein present a case of multiple myeloma (MM) with complicated coagulopathy. Initially, the patient showed severe bleeding tendency due to concomitant acquired hemophilia A and acquired von Willebrand syndrome. Interestingly, the patient also exhibited hyperactivation of factor IX. During treatment for MM, the bleeding complications were ameliorated; however, the patient had central retinal vein occlusion...
July 6, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29920621/hemostasis-and-thrombosis-in-the-oldest-old
#6
Inna Tzoran, Ron Hoffman, Manuel Monreal
There is a growing proportion of the elderly population in the Western world, and these individuals require special considerations regarding a broad variety of aspects, including treatment approaches to illnesses that affect all age groups. The hemostatic system in individuals changes considerably with aging. Specifically, changes in levels of procoagulant and natural anticoagulant factors along with thrombopathy simultaneously create a hypercoagulable state and hemostatic difficulties. Underlying morbidities, such as congestive heart failure, chronic obstructive pulmonary disease, diabetes mellitus, and cancer, increase the risk for venous and arterial thrombosis...
October 2018: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29908036/acquired-von-willebrand-syndrome-in-congenital-heart-disease-surgery-results-from-an-observational-case-series
#7
V Icheva, M Nowak-Machen, U Budde, K Jaschonek, F Neunhoeffer, M Kumpf, M Hofbeck, C Schlensak, G Wiegand
Essentials Bleeding complications during congenital heart disease surgery in neonatal age are very common. We report the perioperative incidence of acquired von Willebrand syndrome (aVWS) in 12 infants. aVWS was detected in 8 out of 12 neonates and infants intraoperatively after cardiopulmonary bypass. Ten patients received von Willebrand factor concentrate intraoperatively and tolerated it well. SUMMARY: Background Cardiac surgery of the newborn and infant with complex congenital heart disease (CHD) is associated with a high rate of intraoperative bleeding complications...
November 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29889319/pediatric-thrombotic-thrombocytopenic-purpura
#8
REVIEW
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (activity <10%), the specific von Willebrand factor (VWF)-cleavage protease. This deficiency may be either acquired (associated anti-ADAMTS13 autoantibodies) or congenital (resulting from biallelic mutations of ADAMTS13 gene). ADAMTS13 deficiency is responsible for the accumulation of high molecular weight multimers of VWF and the formation of platelet thrombi in the microcirculation...
October 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29866817/von-willebrand-factor-regulation-of-blood-vessel-formation
#9
REVIEW
Anna M Randi, Koval E Smith, Giancarlo Castaman
Several important physiological processes, from permeability to inflammation to hemostasis, take place at the vessel wall and are regulated by endothelial cells (ECs). Thus, proteins that have been identified as regulators of one process are increasingly found to be involved in other vascular functions. Such is the case for von Willebrand factor (VWF), a large glycoprotein best known for its critical role in hemostasis. In vitro and in vivo studies have shown that lack of VWF causes enhanced vascularization, both constitutively and following ischemia...
July 12, 2018: Blood
https://www.readbyqxmd.com/read/29743402/-durable-remission-attained-with-rituximab-therapy-in-a-patient-with-acquired-von-willebrand-syndrome-associated-with-cd20-positive-lymphoproliferative-disorder
#10
Honami Kurahashi, Yoshinari Kawabata, Yoshihiro Michishita, Atsushi Kitabayashi, Takahiro Kobayashi, Akihiro Kitadate, Naoto Takahashi
A 61-year-old female with no history of bleeding was admitted to our hospital owing to persistent bleeding after the left knee joint injection and activated partial thromboplastin time prolongation. Subsequent coagulation tests revealed a critically declined level of the von Willebrand factor (VWF) antigen (<10%) and activity (<10%) measurement besides a significantly declined factor VIII activity (4%). Despite diagnosing her with acquired von Willebrand syndrome (AvWS) and managing her bleeding with desmopressin acetate hydrate (DDAVP), we could not precisely make a definitive diagnosis the underlying disorder...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29564686/ofatumumab-for-acute-treatment-and-prophylaxis-of-a-patient-with-multiple-relapses-of-acquired-thrombotic-thrombocytopenic-purpura
#11
Hanny Al-Samkari, Rachael F Grace, Jean M Connors
Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder resulting in potentially life-threating systemic thrombotic microangiopathy due to production of antibodies directed against the von Willebrand factor-cleaving protease ADAMTS13. Typically managed with plasma exchange, glucocorticoids, and the first-generation anti-CD20 monoclonal antibody rituximab, patients with multiple relapses or refractory disease present unique management challenges. We describe a case of a young woman with multiple relapses of TTP despite standard therapy who was treated with ofatumumab, a second-generation anti-CD20 monoclonal antibody, after developing a severe hypersensitivity reaction to rituximab precluding its use...
July 2018: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/29403324/pharmacokinetics-based-clinical-management-of-acquired-von-willebrand-syndrome-a-case-report
#12
Candice M Baldeo, Candido E Rivera, Han W Tun, Prakash Vishnu
von Willebrand disease (VWD) is a common bleeding disorder caused by defective or low levels of von Willebrand factor (VWF). Although most cases of VWD are caused by genetic mutations, some are acquired due to various disease states. In managing VWD, the aim is to normalize plasma levels of both VWF and factor VIII (FVIII), as this aids in hemostasis. Desmopressin usually corrects VWF level in type 1 VWD by inducing the release of endogenous VWF. In cases where desmopressin is ineffective or cannot be used, transfusion of virally inactivated, plasma-derived VWF/FVIII concentrate or infusion of recombinant VWF (Vonvendi) is indicated...
2018: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28987857/intracranial-hemorrhage-in-patients-with-durable-mechanical-circulatory-support-devices-institutional-review-and-proposed-treatment-algorithm
#13
Wyatt L Ramey, Robyn L Basken, Christina M Walter, Zain Khalpey, G Michael Lemole, Travis M Dumont
BACKGROUND: Spontaneous intracranial hemorrhage (ICH) is frequently managed in neurosurgery. Patients with durable mechanical circulatory support devices, including total artificial heart (TAH) and left ventricular assist device (LVAD), are often encountered in the setting of ICH. Although durable mechanical circulatory support devices have improved survival and quality of life for patients with advanced heart failure, ICH is one of the most feared complications following LVAD and TAH implantation...
December 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28759473/novel-therapeutic-approaches-for-thrombotic-thrombocytopenic-purpura
#14
REVIEW
Yvette C Tanhehco, Gowthami Arepally, Ara Metjian
PURPOSE OF REVIEW: Acquired thrombotic thrombocytopenic purpura is an immune-mediated thrombotic microangiopathy caused by antibodies to ADAMTS13 (A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13). Standard treatment with therapeutic plasma exchange and immunosuppression with steroids results in high remission and low mortality rates. However, a number of patients remain refractory to frontline therapy and/or experience multiple relapses. This study reviews emerging therapies for thrombotic thrombocytopenic purpura...
November 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28645643/management-of-thrombotic-thrombocytopenic-purpura
#15
REVIEW
P Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs, typically within the first days of management. In this context, the introduction of rituximab has been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, and increasingly as frontline therapy, with high response rates in the following weeks...
September 2017: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://www.readbyqxmd.com/read/28554655/hemorrhagic-angiodysplasia-of-the-digestive-tract-pathogenesis-diagnosis-and-management
#16
REVIEW
Aymeric Becq, Gabriel Rahmi, Guillaume Perrod, Christophe Cellier
No abstract text is available yet for this article.
November 2017: Gastrointestinal Endoscopy
https://www.readbyqxmd.com/read/28512017/a-case-of-autoimmune-severe-acquired-von-willebrand-syndrome-type-3-like
#17
Chakri Gavva, Prapti Patel, Yu-Min Shen, Eugene Frenkel, Ravi Sarode
Von Willebrand disease (VWD) is the most common congenital bleeding disorder and is due to quantitative or qualitative defects of von Willebrand factor (VWF). Acquired defects of VWF, termed acquired von Willebrand syndrome (AVWS), are due to a host of different mechanisms. Autoantibody-mediated AVWS may be associated with lymphoproliferative or immunological disorders, such as systemic lupus erythematosus (SLE). A large majority of AVWS cases are type 1 or type 2A-like and patients tend to have a mild to moderate bleeding tendency...
June 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28480350/therapeutic-efficacy-of-the-platelet-glycoprotein-ib-antagonist-anfibatide-in-murine-models-of-thrombotic-thrombocytopenic-purpura
#18
Liang Zheng, Yingying Mao, Mohammad S Abdelgawwad, Nicole K Kocher, Mandy Li, Xiangrong Dai, Benjamin Li, X Long Zheng
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal blood clot disorder, is primarily caused by severe deficiency of plasma ADAMTS13 activity resulting from acquired autoantibodies. Plasma exchange is the only effective initial therapy. However, the high mortality rate and the complications associated with plasma exchange therapy remain a major concern. To address unmet clinical needs, therapeutic efficacies of anfibatide, a snake venom-derived platelet glycoprotein Ib antagonist, in murine models of spontaneous thrombocytopenia and shigatoxin-induced TTP were determined...
November 29, 2016: Blood Advances
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#19
REVIEW
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving protease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies, but rarely, it is inherited via mutations of the ADAMTS13 gene...
May 25, 2017: Blood
https://www.readbyqxmd.com/read/28296884/increased-von-willebrand-factor-decreased-adamts13-and-thrombocytopenia-in-melioidosis
#20
Emma Birnie, Gavin C K W Koh, Ester C Löwenberg, Joost C M Meijers, Rapeephan R Maude, Nicholas P J Day, Sharon J Peacock, Tom van der Poll, W Joost Wiersinga
BACKGROUND: Melioidosis, caused by bioterror treat agent Burkholderia pseudomallei, is an important cause of community-acquired Gram-negative sepsis in Southeast Asia and Northern Australia. New insights into the pathogenesis of melioidosis may help improve treatment and decrease mortality rates from this dreadful disease. We hypothesized that changes in Von Willebrand factor (VWF) function should occur in melioidosis, based on the presence of endothelial stimulation by endotoxin, pro-inflammatory cytokines and thrombin in melioidosis, and investigated whether this impacted on outcome...
March 2017: PLoS Neglected Tropical Diseases
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